ADULT T-CELL LEUKEMIA (ATL) WITH AN UNUSUAL IMMUNOPHENOTYPE AND A HIGH CELLULAR PROLIFERATION RATE

A patient with adult T-cell leukemia (ATL) characterized by a suppress or phenotype is reported. A 52-year-old mulatto male presented with sy mptoms and signs of hypercalcemia. His laboratory finding disclosed a peripheral blood specimen with abnormal cells characterized by a rathe r pleomorphic morphology and polylobated nucleous typical of ATL cells . Serum calcium and LDH were 18,2 mg/dl and 1373 IU, respectively. The phenotype of these cells was CD2+, CD4-, CD8+, CD28+ associated with the expression of activated antigens such as CD25, CD38, CD71 and CD30 . Ki-67 positive were found in 20% of cells. The argyrophilic stain fo r nuclear organizer regions (AgNORs) was shown one cluster in 35% of a bnormal cells. The serum antibodies were positive against human T-cell lymphotropic virus type I (HTLV-I) and clinical features were compati ble with the diagnosis of ATL acute type. The combination therapy with cyclophosphamide, vincristine, prednisone decreased the number of leu kemic cells but the clinical course was aggressive. He only responded transiently to treatment and died of multiorgan failure due to uncontr ollable septicemia two weeks after admission.